INCIDENCE OF DEVELOPMENT OF FACTOR-VIII AND FACTOR-IX INHIBITORS IN HEMOPHILIACS
INCIDENCE OF DEVELOPMENT OF FACTOR-VIII AND FACTOR-IX INHIBITORS IN HEMOPHILIACS
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DOI：
10.1016/0140-6736(92)90874-3
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发表时间：
1992-03-07
期刊：
影响因子：
168.9
通讯作者：
KORNHUBER, B
中科院分区：
文献类型：
--
作者：
EHRENFORTH, S;KREUZ, W;KORNHUBER, B
因子VIII：C抑制剂的发展仍然是血友病A患者反复输血的最严重的并发症之一。据报道，受影响的患者比例从3.6%到25%不等，但这些数字主要来自回顾数据和已知血友病患者的总数，而不是真正有风险的人数。这里的评估是基于1976年开始的一项前瞻性研究，该研究是关于1970年以后出生的血友病患者中FVIII或FIX活性为5%或更低且至少接受过一次替代治疗的血友病患者中出现抑制剂的发生率。63名血友病A儿童中46名和17名血友病B儿童中13名符合登记标准。抑制物仅在以前接受FVIII产品治疗的血友病A患者中出现--抑制物浓度高的有12例，低的有3例。24%(15/63)的血友病A患者和52%(14/27)的重症患者出现了抑制物。所有血友病患者的抑制物发生率为每1000人中39.1人-年的观察。所有的抑制剂都是在0.08-5.2岁的患者中首次检测到的。在6岁时，累积风险为33%。研究结果表明，以前的报告低估了获得FVIII抑制剂的风险。为了评估这种并发症的真实风险，需要进行前瞻性、标准化的研究，特别是在儿童中。
The development of factor VIII:C inhibitors remains one of the most serious complications of repeated transfusion in patients with haemophilia A. The proportion of patients affected has been reported to range from 3.6% to 25%, but these figures have been derived mainly from retrospective data and from total numbers of known haemophiliacs instead of number at true risk. The assessment here is based on a prospective study, started in 1976, on the incidence of inhibitor development in haemophiliacs born after 1970 whose FVIII or FIX activity was 5% or less, and who had received replacement therapy at least once.46 of 63 children with haemophilia A and 13 of 17 with haemophilia B fulfilled the enrolment criteria. Inhibitors developed only in haemophilia A patients who had previously been treated with FVIII products-inhibitor concentrations were high in 12 and low in 3. Inhibitors developed in 24% (15/63) of all haemophilia A patients, and in 52% (14/27) of those with severe disease. The incidence of inhibitor development for all haemophilia patients was 39.1 per 1000 patient-years of observation. All inhibitors were first detected when patients were aged 0.08-5.2 years. The cumulative risk was 33% at age 6 years.The findings indicate that previous reports have underestimated the risk of acquiring FVIII inhibitors. Prospective, standardised studies, especially in children, are needed for the assessment of the true risk of this complication.